GSE138493 Microarray expression profiling in zebrafish rnaset2 mutant

Contributors : No émie Hamilton ; Jutta GaertnerSeries Type : Expression profiling by arrayOrganism : Danio rerioRNASET2-deficient leukoencephalophathy is a severe leukodystrophy affecting children with psychomotor impairements in their forst year of life. We generated the first zebrafish model for a human leukodystrophy by targetting the ortholog rnaset2 gene using mutagenesis. This zebrafish mutant recapitulated the human clinical manisfestations and developed white matter defects detectable by MRI. Additionally, this zebrafish mutant identified this disease as a lysosomal storage disorders, with RNA accumulating in neurons. To understand how accumulation of RNA in neurons trigger white matter lesions, we undertook an unbiased approach and perfored microarray analysis. We identified differentially expressed genes in mutants and the immune system as a key pathway disregulated in the zebrafish rnaset2 mutant.
Source: GEO: Gene Expression Omnibus - Category: Genetics & Stem Cells Tags: Expression profiling by array Danio rerio Source Type: research